Question

What is the pathophysiology of acute sickle cell disease (SCD) crisis and why is pain the predominate feature of acute crises?  

Explain the genetics of hemophilia

Describe the pathophysiology of hydrocephalus in infants with myelomeningocele.

How does lead poisoning account for the child’s symptoms?

What is thought to be the pathophysiology of idiopathic scoliosis?

Answer 1

1. The pathophysiology of Acute sickle cell disease crisis is:

Sickle celled RBC  Vaso occlusive crisis Reduced blood flow to vital organs Pain, Ischemia, Necrosis

Pain is the predominant feature of acute sickle cell disease crisis. The sickle celled RBCs cause vaso occlusive crisis . This reduce blood flow to vital organs and results in tissue damage and other changes in cell. This causes pain. Inshort vaso occlusive crisis causes pain in achte sickle cell disease crisis.

2. Hemophilia is called 'X' linked or sex linked recessive disorder. The X chromosome is responsible for this disorder. Males are often affected by hemophilia as they have only one X chromosome, which is inherited from their mothers. . Females are affected when both the X chromosomes are having genes for hemophilia.

3.Pathophysiology of hydrocephalus in infants with myelomeningocele:

Myelomeningocele is also referred to as Spinal bifida. The spinal bifida disturbs and obstructs the normal flow of fluid out of the brain. This results in the excess of Cerebrospinal fluid(CSF) in the brain and causes hydrocephalus.

4.The common symptoms shown by lead poisoning in children are irritability, loss of appetite, wieghtloss, anaemia, fatigue etc. Lead poisoning can also result in growth, behavioural and intellectual problems.

5.The pathophysiology of Idiopathic scoliosis can be said as

Idiopathic causes Vertebral abnormalities Curvature of spine greater than 10⁰ Idiopathic scoliosis.